Articles

Mitochondrial Disease and Mitochondrial Myopathies: what are they?

What is a Mitochondrial Disease?

Imagine a major city with half its power plants shut down. At best, such conditions would produce a “brown out” with large sections of the city working far below optimum efficiency. Now imagine your body working with one-half of its energy producing facilities shut down. The brain would be impaired, vision would be dim, muscles would twitch spastically or would be too weak to allow your body to walk or write, your heart would be weakened, and you would not be able to eat and digest your food. For large numbers of people, especially children, this is precisely the situation in which they find themselves due to the defects in the mitochondria organelles found in almost every cell of the body which are responsible for the body’s energy production. Mitochondrial diseases compromise their lives and can be fatal. (Thanks to the UMDF -- The United Mitochondrial Disease Foundation -- for this description of Mitochondrial Disease -- for more info on their work, please "click" on the UMDF link at the bottom of this page)

Mitochondrial myopathies are a group of neuromuscular diseases caused by damage to the mitochondria - small, energy-producing structures found in almost all cells in the body that serve as the cells' "power plants." Nerve cells in the brain, muscle cells and heart cells require a great deal of energy, and thus appear to be particularly damaged when mitochondrial dysfunction occurs. Some of the more common mitochondrial myopathies include Kearns-Sayre syndrome, myoclonus epilepsy with ragged-red fibers (MERRF), and mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes (MELAS) and Friedreich's Ataxia. The symptoms of mitochondrial myopathies include muscle weakness or exercise intolerance, heart failure or rhythm disturbances, dementia, movement disorders, stroke-like episodes, deafness, blindness, droopy eyelids, reduced ability of the eyes to move, vomiting, and seizures. The disorders range in severity from progressive weakness to death. The age of onset ranges from birth to adulthood, with the majority of cases occurring before the age of 20. Exercise intolerance or muscle weakness usually develops by the age of 20. During physical activity, muscles may become easily fatigued or weak. Muscle cramping may rarely occur. Nausea, headache, and breathlessness are also sometimes associated with mitochondrial myopathies.

What is the prognosis?

The prognosis for patients with mitochondrial myopathies varies greatly from patient to patient because disease progression depends largely on the type of disease and the degree of involvement of various organs.

Are there any treatments?

Although there is no specific treatment for any of the mitochondrial myopathies, physical therapy may extend the range of movement of muscles and improve dexterity. Vitamin therapies such as riboflavin, coenzyme Q10, vitamins C and K, Thiamine, Alpha Lipoic Acid and L-carnitine may improve function in some patients. If you are considering vitamin therapies discuss the issue first with your health care professional. If you get the go-ahead and CoQ10 is recommended, we suggest you start with our Q-Gel® CoQ10.

Why Q-Gel® CoQ10 from Tishcon?

Because, Q-Gel® CoQ10 is formulated via a bioavailability enhancing patented technology which renders the insoluble CoQ10 duo-soluble -- it can dissolve in both fat and water!Clinical studies, as well as in-vitro cell culture studies have documented the absolute superiority of Q-Gel CoQ10 over other non-solubuilized CoQ10 products. Note that we also have bio-enhanced CoQ10 from Tishcon available in liquid in a bottle for those patients who cannot swallow a soft gel.

Children’s Q-Gel® CoQ10 Dosages:

Normally healthy children should NOT need to supplement their diet with CoQ10. Dosages for children with mitochondrial diseases and other conditions that could be helped with Q-Gel CoQ10 should be discussed with your child’s Doctor. Per our senior scientist, a starting point you may want to discuss would be 3 mg. per pound of the child’s body weight. For example, if the child weighs 20 pounds, start with 20 X 3 mg = 60 mg per day in divided doses, i.e. 20 mg 3 times daily.